The unicuspid aortic valve is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Other congenital disorders may accompany this phenomenon and aortic dilatation and left ventricular hypertrophy are frequent complications. We present a case report of a young, symptomatic patient with a unicuspid aortic valve, complicated by dilatatio...

Congenital aortic valve anomalies are quite a rare finding in echocardiographic examinations. A case of a 19 year old man with a pentacuspid aortic valve without aortic stenosis and regurgitation, detected by transoesophageal echocardiography, is presented.

Fetal echocardiography has yet to have an impact on the treatment of congenital heart disease. Critical aortic valve stenosis was diagnosed by echocardiography before birth in a 35 week gestation fetus. The risks to the fetus and mother associated with prolonged rupture of membranes prompted their transport to a hospital with cardiac surgical and high-risk perinatal facilities. Prenatal and pos...

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenge...

To examine factors that might affect progression in valvular aortic stenosis (AS), we reviewed serial hemodynamic studies in 50 adult patients. Seven patients had congenital, 22 rheumatic and 21 degenerative-calcific aortic stenosis. The patients with calcific aortic stenosis were older (62 ± 6 vs 51 ± 9 years, p < 0.001) and had onset of murmur later in life. For all patients average values at...

splenomegaly-induced thrombocytopenia is fully described in hematological and surgical literature, but its association with severe aortic stenosis is rare. we present a case of severe aortic valve stenosis with severe splenomegaly-induced thrombocytopenia in which aortic valve replacement was done with a number 23 homograft and splenectomy was performed after the end of cardiopulmonary bypass. ...